Presentation
Early presentation and prompt treatment of anorectal malformations is important to prevent such potential life threatening complication. [ncbi.nlm.nih.gov]
Female Demographics and Presentation Table 2. Male Demographics and Presentation Careful physical examination confirmed the diagnosis in all the cases. Typically the perineum was raw and irritated. [pediatrics.aappublications.org]
Although, female ARM and male children with low ARM may present late, almost all male patients with high ARM present within first few days of life. [atmph.org]
On perineal examination, an anocutaneous fistula with meconium was present between the scrotum and well-formed anal pit [Figure 1]. Erect abdominal X-ray revealed pneumoperitoneum [Figure 2]. [jcnonweb.com]
Entire Body System
- Pain
The patient presented with intermittent diffuse abdominal pain at the emergency department. Physical examination revealed distended abdomen with hyperactive bowel sounds and without obvious tenderness or rebounding pain. [e-fjs.org]
Of these, 10 reported abdominal pain 1 to 3 times per week and 9 reported pain 1 to 2 times per month. This was equally frequent in patients with a past history of either high/intermediate or low malformations. [intechopen.com]
We will keep the child as comfortable as possible, by giving regular pain relieving medications. [gosh.nhs.uk]
We will keep your child as comfortable as possible, by giving regular pain relieving medications. [amardeepchildrenhospital.com]
523 Chronic Nonspecific Diarrhea of Childhood 524 Gastrointestinal Bleeding 525 Peptic Ulcer Disease 527 Intussusception 529 Oral Rehydration Therapy 531 Anorectal Malformations 534 Ulcerative Colitis 535 Crohns Disease 538 Chronic Recurrent Abdominal Pain [books.google.com]
- Surgical Procedure
This surgical procedure creates a stoma, an opening on the abdomen through which stool passes. [childrenscolorado.org]
The colostomy is a surgical procedure that brings a segment of the large intestine out through an opening called a stoma that is made in the abdominal wall; this is a temporary situation and the colostomy is closed a few weeks after pull-through surgery [chp.edu]
It may take several years to determine the final outcome of the surgical procedure. Specialized diets and medications may be needed to help your child have regular bowel movements and to reduce the chance of incontinence or soiling. [eapsa.org]
All the surgical procedures were uneventful and at present the patient is doing well and is on regular follow-up. [jemds.com]
Selecting the one- or three-step surgical procedure to treat the disease, especially in female neonates with rectovestibular fistula, is a subject of debate. This study aims to compare the advantages and disadvantages of these two methods. [afrjpaedsurg.org]
- Weight Gain
The patient was then free of constipation and achieved an improved nutritional status with appropriate weight gain. [e-fjs.org]
Delayed presenters also have inadequate weight gain and increased parental anxiety for a greater period of time [4,5]. Anterior or posterior sagittal anorectoplasty has been the operation of choice in terms of long-term anorectal function [6]. [casereports.in]
It might be an easier surgical technique because of the delay in definite repair and weight gain by the infant. [afrjpaedsurg.org]
Three months later, with good weight gain (patient weight-9kg), the child being on a healthy mixed diet with good bowel decompression through the stoma, a posterior sagittal anorectoplasty (PSARP) was done. [atmph.org]
- Swelling
There may be abdominal swelling caused by a blocked intestine. A baby born with this condition can have other medical difficulties as well, especially in the genitals, bladder, urethra, heart, kidneys, esophagus, limbs or spine. [pullthrough.org]
When he’s unable to have a bowel movement, his belly swells and becomes so distended that often his shirts no longer fit. In the six months since Bennett had solids added to his diet, he has been hospitalized for fecal impactions five times. [complexchild.org]
As the faeces can only be partially removed from the body, this will not relieve the pressure in the abdomen, causing swelling and vomiting. [gosh.nhs.uk]
- Trisomy 21
21 (Down syndrome) Management The radiological evaluation of a newborn with imperforate anus should include an abdominal and pelvic ultrasound to evaluate for genitourinary defects as they need to be dealt with in the newborn period to avoid serious [humpath.com]
This increases in patients with trisomy 21 and ARM, in whom 95% have no fistula [32]. [jpss.eu]
Gastrointestinal
- Vomiting
You should call your family doctor (GP) or the ward if the child: has serious abdominal pain and/or diarrhoea has serious constipation Seek urgent medical help if the child: vomits when feeding has a swollen stomach vomits green liquid is not gaining [gosh.nhs.uk]
This enables us to keep the stomach empty so the baby does not vomit and your baby will be given fluid through a drip sited in one of its veins. [uhs.nhs.uk]
Delay in diagnosis of ARM may lead to early complications like abdominal distension, vomiting, dehydration, intestinal perforation, respiratory distress, sepsis and even death. [atmph.org]
Clinical presentation in neonates and older children Neonate Older Child Failure to pass meconium in first 24-28 hours life Chronic constipation Abdominal distension Chronic abdominal distension Bilious vomiting Vomiting Neonatal enterocolitis/ sepsis [jpss.eu]
Also call your doctor if your child goes more than two days without having a bowel movement or has vomiting, fevers or abdominal pain. [eapsa.org]
- Abdominal Distension
Delay in diagnosis of ARM may lead to early complications like abdominal distension, vomiting, dehydration, intestinal perforation, respiratory distress, sepsis and even death. [atmph.org]
Meconium and air were released, which decreased the abdominal distension. [saudijgastro.com]
Clinical presentation in neonates and older children Neonate Older Child Failure to pass meconium in first 24-28 hours life Chronic constipation Abdominal distension Chronic abdominal distension Bilious vomiting Vomiting Neonatal enterocolitis/ sepsis [jpss.eu]
In view of the abdominal distension, an ascending loop colostomy was done as the initial procedure. [jemds.com]
Abdominal distension was present. All the patients were operated by cut back anoplasty. The patients are in regular follow-up and anal dilation. Figure 1 Figure 1: Showing low anorectal malformation in a female without fistula. [ispub.com]
- Failure to Thrive
Failure to thrive Colonic atresia Enterocolitis Table 2. [jpss.eu]
Cardiovascular
- Heart Disease
425 Mitral Valve Prolapse 427 Anomalous Origin of the Left Coronary Artery 429 Cardiomyopathy 433 Infective Endocarditis 439 Myocarditis 444 Rheumatic Heart Disease 447 Aortic Arch and Pulmonary Artery Abnormalities Vascular Rings and Slings 452 Diseases [books.google.com]
[…] failure心不全 heart rate心拍数 heart sound心音 hemangioma血管腫 hemolytic anemia溶血性貧血 hemolytic disease due to Rh isoimmunizationRh不適合による溶血性疾患 hereditary spherocytosis (HS)遺伝性球状赤血球症 heterozygoteヘテロ(異型)接合体 high risk infantハイリスク児 Hirschsprung diseaseヒルシュスプルング病、先天性巨大結腸症 [tokyo-med.ac.jp]
Psychiatrical
- Suggestibility
Histopathology of the tissue specimen was suggestive of mucormycosis. Postoperatively, he received intravenous amphotericin B (liposomal) and was started on liquid enteral nutrition after 2 weeks. [ncbi.nlm.nih.gov]
A day 1 neonate was referred with features suggested of peritonitis. [saudijgastro.com]
However, recent reports suggest that tethered cord is as common in patients with a low lesion as in those with a high lesion. [keio.pure.elsevier.com]
Nevertheless, soiling is a common complication. [7] Recently, Borg et al. described the long-term follow-up of ARMs with megarectosigmoids, [1] suggesting that early implementation of bowel management was helpful in normalizing the bowel function and [e-fjs.org]
Many systems of classification have been suggested; however, the terms high and low have been broadly recognized and applied worldwide. [ispub.com]
Treatment
The results of the conservative treatment were comparable with that of the surgical treatment. Here, we presented the case of a 55-year-old man. Megarectosigmoids have been rarely documented in patients of this age. [e-fjs.org]
Early presentation and prompt treatment of anorectal malformations is important to prevent such potential life threatening complication. [ncbi.nlm.nih.gov]
Our renowned surgeons and faculty continue to push the frontiers of surgical science through innovative treatment and leading-edge research, changing lives and the future of surgical treatment, each and every day. [medicine.yale.edu]
The ultimate judgement regarding a particular clinical procedure or treatment must be made by the clinician in the light of the clinical data presented by the patient and the diagnostic or treatment options available. [uhs.nhs.uk]
Prognosis
Of the patients, 83% showed good prognosis and 15% had fair and poor prognosis. Our findings are similar. In the study of Hassink, there was a positive correlation between prognosis improvement and increasing age 15. [journals.lww.com]
Patients with this defect have all the necessary elements to be continent and have an excellent functional prognosis. [slideplayer.com]
These studies help to establish initial treatment and prognosis. How is ARM treated? All children with an ARM need at least one operation called an anorectoplasty or “pull-through”. [chp.edu]
Nonetheless, the prognosis of affected patients is far from excellent, as some degree of constipation is a frequent complaint in the long-term follow-up. Constipation in "low" ARM has been reported in 42%-70% of cases. [ncbi.nlm.nih.gov]
Despite this, the prognosis after surgery is very good, though children who’ve had this condition may suffer from constipation or urinary incontinence. [pullthrough.org]
Etiology
2016/5/4/274/194177 Introduction Neonatal colonic perforations are rare and even rarer are those which complicate anorectal malformations (ARMs). [1], [2] The commonly described etiologies of colonic perforations are intestinal atresias, Hirschsprung's [jcnonweb.com]
Vascular accidents occurring in utero are most commonly implicated in the etiology of CA; whereas, the etiology of ARM is widely considered multifactorial and may include participation of recessive genes (Nazer et al. 2000 ;Mo et al. 2001 ;Shinha et al [springerplus.springeropen.com]
Head Trauma 234 Sepsis and Septic Shock 242 Nonpolio Enteroviruses 332 Respiratory Syncytial Virus 334 Rhinoviruses 336 Roseola and Human Herpesviruses 337 Rubella 338 Rubeola 339 VaricellaZoster Virus 342 Viral Gastroenteritis 344 Disorders of Unknown Etiology [books.google.com]
BACKGROUND The etiology and pathogenesis of anorectal malformations (ARM) are multifactorial, poorly understood and controversial. [research.unipd.it]
Two lateral folds of cloacal tissue join the urorectal septum to complete the separation of the urinary and rectal tracts. 4 5 Imperforate anus Includes agenesis and atresia of the rectum and anus Etiology: unknown Incidence: 1 in 4,500 SEX: 60% male [slideplayer.com]
Epidemiology
"Genetics, Pathogenesis and Epidemiology of Anorectal Malformations and Caudal Regression Syndrome". In Holschneider, Alexander Matthias; Hutson, John M. (eds.). [en.wikipedia.org]
Springer, Berlin Heidelberg New York, pp 3–15 Google Scholar Harris J, Källén B, Robert E (1995) Descriptive Epidemiology of Alimentary Tract Atresia. [link.springer.com]
Epidemiology: Anorectal malformations (ARM) occurs in 1 baby in every 5,000 live births. It is equally seen in boys and girls. Figure 1: Picture of a baby's bottom without an anal opening. Picture courtesy of MJArca 11/2016. [eapsa.org]
Anal atresia, vertebral, genital and urinary tract anomalies: A primary polytopic developmental field defect identified through an epidemiological analysis of associations. Am J Med Genet 2000;13:95(2):169-73. 10. [pjms.com.pk]
[…] defects were seen less commonly in our patients. [1], [2], [17] Postoperative wound infection occurred in 3% of the patients, major source being contamination during colostomy as reported by others also. [7], [10], [18] This report gives an outline of the epidemiological [jiaps.com]
Pathophysiology
[…] surgery for low ARM can be done at the same time. [1] The overall mortality of perforated ARM is 19%. [1] Sepsis and disseminated intravascular coagulation were frequent causes of death. [1] Early diagnosis of ARM in neonates, better understanding of the pathophysiology [jcnonweb.com]
It is likely that other pathophysiological “triggers” tip the balance in favour of heavy soiling. Rapid colonic transit may be one such trigger. [intechopen.com]
Keywords: constipation, paediatric colorectal, Hirschsprung disease, anorectal malformation, VACTERL Hirschsprungs disease Definition and pathophysiology Hirschprungs disease (HD) is a congenital condition characterised by the absence of ganglion cells [jpss.eu]
Prevention
Early presentation and prompt treatment of anorectal malformations is important to prevent such potential life threatening complication. [ncbi.nlm.nih.gov]
This should be preventable by passing the dilator daily for the first few months of life. In the longer term constipation may be a problem but this can usually be managed by diet or medication. [uhs.nhs.uk]
In this case anal dilation will be all that’s necessary and surgery can be prevented, unless the anus is in the wrong place. [pullthrough.org]
Thus, the posterior muscle complex surrounds the rectum and helps prevent rectal prolapse. [22] (See the images below.) Operative view of posterior sagittal anoplasty in newborn with rectoperineal fistula. [emedicine.medscape.com]
We provide a Bowel Management Program, where we work closely with you and your child to assess function and provide a way for your child to stay clean of stool and prevent accidents. Learn More > [intermountainhealthcare.org]